Reactome | Mitochondrial BTD hydrolyses BCTN

Mitochondrial BTD hydrolyses BCTN

Stable Identifier

R-HSA-4167509

Type

Reaction [transition]

Species

Homo sapiens

Compartment

mitochondrial matrix

ReviewStatus

5/5

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Human biotinidase (BTD, EC 3.5.1.12) (Cole et al. 1994) catalyzes the hydrolysis of biocytin (BCTN, aka biotinyl-lysine), a product of biotin dependent carboxylase degradation, to biotin (Btn) and lysine. As a result, Btn is again available to be used in the biotinylation of apo-carboxylases in the mitochondrion. BTD is both secreted from various cells and localised in the mitochondria (Wolf & Jensen 2005). BTD deficiency, an autosomal recessive disorder, results in a secondary Btn deficiency that leads to juvenile onset multiple carboxylase deficiency (MIM:253260) (Wolf et al. 1983).

Literature References

PubMed ID	Title	Journal	Year
16150625	Evolutionary conservation of biotinidase: implications for the enzyme's structure and subcellular localization Wolf, B, Jensen, K	Mol. Genet. Metab.	2005
7509806	Human serum biotinidase. cDNA cloning, sequence, and characterization Cole, H, Reynolds, TR, Lockyer, JM, Buck, GA, Denson, T, Spence, JE, Hymes, J, Wolf, B	J. Biol. Chem.	1994
6883721	Biotinidase deficiency: the enzymatic defect in late-onset multiple carboxylase deficiency Wolf, B, Grier, RE, Allen, RJ, Goodman, SI, Kien, CL	Clin. Chim. Acta	1983